Anti-acid therapy in SSc-associated interstitial lung disease: long-term outcomes from the German Network for Systemic Sclerosis

Science and Research

OBJECTIVES: Gastroesophageal reflux disease (GERD) occurs frequently in patients with systemic sclerosis (SSc). We investigated whether the presence of GERD and/or the use of anti-acid therapy, specifically proton-pump inhibitors (PPIs), are associated with long-term outcomes, especially in SSc-associated interstitial lung disease (SSc-ILD). METHODS: We retrospectively analysed patients with SSc and SSc-ILD from the German Network for Systemic Sclerosis (DNSS) database (2003 onwards). Kaplan-Meier analysis compared overall survival (OS) and progression-free survival (PFS) in patients with GERD vs without GERD (SSc and SSc-ILD), and PPI vs no PPI use (SSc-ILD only). Progression was defined as a decrease in either % predicted forced vital capacity ≥10% or single-breath diffusing capacity for carbon monoxide ≥15%, or death. RESULTS: 2,693/4,306 (63%) registered patients with SSc and 1,204/1,931 (62%) with SSc-ILD had GERD. GERD was not associated with decreased OS or PFS in patients in either cohort. In SSc-ILD, PPI use was associated with improved OS vs no PPI use after 1 year (98.4% [95% confidence interval: 97.6-99.3]; n = 760 vs 90.8% [87.9-93.8]; n = 290) and after 5 years (91.4% [89.2-93.8]; n = 357 vs 70.9% [65.2-77.1]; n = 106; P< 0.0001). PPI use was also associated with improved PFS vs no PPI use after 1 year (95.9% [94.6-97.3]; n = 745 vs 86.4% [82.9-90.1]; n = 278) and after 5 years (66.8% [63.0-70.8]; n = 286 vs 45.9% [39.6-53.2]; n = 69; P< 0.0001). CONCLUSION: GERD had no effect on survival in SSc or SSc-ILD. PPIs improved survival in patients with SSc-ILD. Controlled, prospective trials are needed to confirm this finding.